Tarlov Cysts and Associated Dural Ectasia-When to Operate

William C. Welch MD, FAANS,FACS, FICS Robert L. Bailey MD, Robert Whitmore MD University of Pennsylvania School of Medicine Donlin Long, MD, FAANS, FACS Johns Hopkins Hospital

First of many water/sea quotes

"To be or not to be that is the question: Whether 'tis nobler in the mind to suffer The slings and arrows of outrageous fortune, Or to take arms against a sea of troubles And, by opposing, end them. William Shakespeare Hamlet.

Case
59 yo female with 4 year hx of LBP, posterior leg pain, and pelvic discomfort (ground glass) Multiple evaluations, multiple physicians Had undergone spinal cord untethering surgery 2 years ago No specific neurological complaints or deficits, suggestion of bladder dysfunction

Thoughts?
Diagnosis? Treatment options? Outcomes? Literature?

Introduction
Definition:
Collections of CSF between the endoneurium and perineurium of the nerve root sheath near the dorsal root ganglion in S1-S4 region.
Microscopic evidence of neural injury

Prevalence estimated to be 4.6% among general adult population

Definition has become liberalized to include all nerve root associated cysts

Frequency: 1-2% of patients have nerve root associated cysts on MRI

Historical Classification
Identified on autopsy specimens in the late 1930s by Tarlov and Rexed
One subsequent autopsy description (NEJM Clinicopathologic Conference, Long) 1950s Tarlov described 8 patients in whom symptoms were attributed to the cysts

Classification
Nabors et al.
Tarlov or perineural cysts are Type II
Nerve tissue in the walls of the cyst, unclear if communicating with perineural arachnoid space

Type I:
Extra-dural cysts without neural involvement, enlarge foramen and scallop vertebral bodies

Type III:
Intradural, either congenital or caused by trauma

Comparison to Other Cysts

Potential communication with SA space Delayed filling in myelograms Found distal to the junction of posterior nerve root and dorsal root ganglion in sacral region Walls contain nerve fibers Often multiple, extending around the circumference of nerve root Communicates freely with SA space Rapid filling in myelograms Found proximal to dorsal root ganglion throughout vertebral column Walls lined by arachnoid mater with no signs of neural elements No pattern of formation with regards to multiplicity

Etiology
Formation:
Inflammation within nerve root cysts followed by inoculation of CSF Congenital origin Arachnoidal proliferation along exiting sacral nerve root Blockage of venous drainage in the perineuria and epineurium secondary to hemosiderin deposition after trauma Collagen vascular disorders
Marfans syndrome

Symptoms
Usually asymptomatic, but may be attributable to symptoms in 15-30% of cases Tendency to increase in size over time, potentially causing complications and eroding sacral bone 4 Categories of symptomatic patients:
Group 1: Pain on tail bone that radiates to legs with potential weakness Group 2: Pain on bones, legs, groin area, sexual dysfunction and dysfunctional bladder Group 3: Pain that radiates from cyst site across hips to lower abdomen Group 4: No pain, only sexual and bladder dysfunction

Symptoms
Wide spectrum of symptoms including:
Back pain, perineal pain, sciatica, cauda equina syndrome, dysuria, urinary incontinence, coccygodynia, sacral radiculopathy, headaches, retrograde ejaculation, parathesia, hyperesthesia, difficulty with ambulation, motor dysfunction in lower extremities, abdominal pain

Symptoms
Etiology of symptoms:
Direct nerve injury Cyst pressure on adjacent nerve roots Transmssion of CSF pressure into cyst
subsequent further compression of nerve root nerve root wall tension

CSF leakage (functional pseudomeningocele) Sacral erosion Unclear etiological postulates

Symptoms
Nerve root symptoms:
Radiculopathy
Typically S1 and S2 May have localized pain at cyst site Can be thoracic, potentially cervical

Sacral and Pelvic pain

Variable pelvic symptoms
pressure, ground-glass, others

Bladder/bowel symptoms Sacral erosions

CSF hypotensive symptoms

Postural headache

Physical Examination
General exam:
Collagen disorder Neurofibromatosis CRPS Radiculopathy
Hypesthesia Decreased reflexes Strength testing Perineal sensation

Postural symptoms

Evaluation
Evaluation
Is there any other potentially reasonable cause of the patients symptoms EMG testing to look for radiculopathy Plain films CT (may wish to include sacrum) Myelogram MRI of lumbar and sacral spine MRI of pelvis Urological studies Gynecological studies

Treatment Options
Do Nothing
Run Away!

Expectant therapy (most common)

PT/OT Epidural steroids Others

Invasive Non-Surgical Therapy Surgical Options

I pass with relief from the tossing sea of cause and theory to the firm ground of result and fact. Winston Churchill

Literature

Neurosurgery. 40(4), April 1997, pp 861-865 3 patient cases in which lumbar drain was placed and resulted in alleviation of symptoms 2/3 underwent lumboperitoneal shunt with resolution of symptoms for 11 and 9 months each Report data supporting the role of the hydrostatic and pulsatile forces of CSF in the symptomatology of the cysts. External lumbar CSF drainage my be used as adjuvant diagnostic tool if doubt exists about resolution of symptoms with intervention Although proposed by the authors as such, not considered a viable long-term treatment option as symptoms recur

Literature
Prevalence and percutaneous drainage of cysts of the sacral nerve root sheath (Tarlov cysts). Paulsen RD, Call GA,
Murtagh FR. AJNR Am J Neuroradiol. 1994 Feb;15(2):293-7
7 cysts were drained in 5 patients using a percutaneous CT-guided aspiration method Instant pain relief lasted from 3 weeks to 6 months Cysts repressurized and the patients' symptoms returned.
Technique can be a quick and simple way of at least attaining a painfree interval

Literature

Authors report a decrease in the intraprocedural severe pain that develops during aspiration, which is thought to be related to the negative pressure retraction on the dura

CT-guided biopsy and aspiration of Tarlov cysts may help in proving the cyst is the cause of the symptoms and guiding appropriate therapy.

Large Tarlov cyst, causing back pain when patient coughed, treated by endoscopic placement of shunt from cyst to peritoneum, with resolution of symptoms.

Literature
AJR February 1997 vol. 168 no. 2 367-370

4 patients treated initially with CT-guided aspiration with recurrent symptoms within 17-28 weeks Results: Improvement or resolution of pain with no recurrence during follow-up of 7-23 month periods Complications: 3/4 patients developed aseptic meningitis Authors postulated that resolution and lack of recurrence of symptoms following fibrin glue injection may be due to stimulation of fibroblasts and subsequent fibrosis that occurs with fibrin glue resorption

Literature

AJNR 2011 32: 1469-1473

AJNR 2011 32: 1469-1473

Literature

AJNR 2011 32: 1469-1473

Literature

AJNR 2011 32: 1469-1473

Surgical Options
Numerous techniques/strategies have been proposed Simple posterior sacral bony decompression has low success rates Microsurgical excision consists of sacral laminectomy or laminoplasty followed be resection of the wall of the cyst
Nervous fibers of the parental nerve roots may lie directly in the walls of the cyst

Suturing walls of the cyst, neck ligation to close communication of the cyst with the subarachnoid space Excising the cyst and sacrificing parental root? Absorbable gelatin sponge and/or fibrin glue and muscle or fat patching to fill the cyst cavity and cover dural defects
Neurologic worsening and cauda equina syndrome have been reported

Literature
Mummaneni et al. Microsurgical Treatment of Symptomatic Sacral Tarlov Cysts. Neurosurgery, 47(1); 2000, p74-79.
Retrospective review of 8 adult patients with radicular pain Performed sacral laminectomies with cyst fenestration and imbrication Closure reinforced with epidural fat or muscle grafts with fibrin glue application. Improvement in symptoms in 7/8, bladder control improved in 2/3. No CSF leaks or new deficits.

Mummaneni

Literature

Literature

Literature

Literature
Yucesoy et al. Filling of a Sacral Bone Defect From a Perineural Cyst by Cementation. JSDT, 15(6), 2002, p. 523-525.
Case Report: Radicular symptoms with Tarlov Cyst Partial excision, cyst imbrication, methylmethacrylate filling of sacral bone defect

Literature
D. Long (in preparation)
456 consecutive patients (90% female, age 27-68 years) referred for evaluation and treatment of perineural cysts 424 patients had identifiable cysts
53% unilateral, single root 37% bilateral, single level 10% bilateral, multiple nerve roots 32 patients had other cysts (dural ectasia and internal meningocele)

Literature (Long, cont.)

220 patients excluded from study due to loss of follow-up at one year, pain generator not felt to be cyst-related, other causes 204 patients included in study (90% female)
113 patients had repeated diagnostic root block with anesthetics of different half-lives 193 patients had pain relief with aspiration of cyst

Literature (Long, cont.)

75% of patients had specific or generalized lower back pain and/or sciatica and/or perineal pain and/or bladder/bowel dysfunction. 42% of patients had decreased perineal sensation/pain with intercourse Follow-up MRI scans (all pts at 1 year, 100 pts at 2 years, 36 pts at 3 yrs and 28 pts at 5 years) demonstrated that 96% unchanged in size

Complications
CSF DRAINAGE!!!
In different series: 1/11, 1/3, 1/13 patients with postop CSF leaks Treatment: prolonged lumbar drainage Some authors have recommended routine postop lumbar drainage for 3-7 days to prevent CSF leakage and allow for graft healing

Nerve root damage during excision

Electrophysiological monitoring recommended by many authors

Personal Experience
23 cases over 21 years (last 12 in 5 years) Approach generally the same
Osteoplastic laminotomy Imbrication of cyst Sealant +/- CSF drainage +/- Paraspinous muscle flap

2 patients no improvement
both sealed well

Case Presentation
59 yo F with severe low back pain, bilateral posterior leg pain radiating into feet. Low sacrum feels like a hot torch. Admits to vaginal cramping, pelvic discomfort PSH: Cord detethering 4 years ago Exam: Neurologically intact, no deficits appreciated

Case Presentation
Previously underwent cyst aspiration followed by fibrin glue injection with alleviation of symptoms for 11 months
Photos courtesy of Dr. Don Koenigsberg, D.O.

Case Presentation
Recent MRI

Case Presentation
Recent MRI

Case Presentation
Intraop

Suggestions?
Surgery included S1-3 osteoplastic laminotomy The dura was redundant, extremely thinned and clearly leaking CSF into the sacral defect Pseudomeningocele? Tarlov cyst? Closed primarily (still leaking), subcutaneous drains, bed rest, paraspinous muscle flap

Results
Ground glass sensation completely resolved Leaked through incision after two weeks

Suggestions?
Surgery included removal of S1-3 osteoplastic laminotomy The dura was re-explored and could not be primarily repaired any better than had been done Plastic surgery closed over 4 drains with paraspinous muscle flaps Subarachnoid drain placed

Outcome
Bed rest for 2 weeks after discharge Dry, satisfied (overall) with results

Conclusions
Relatively broad morphological criteria Similar symptoms Most appropriately not treated Interventional radiology treatments provide some prognostication Surgery is customized, involved Generally speaking, outcomes good

Thank you for your attention. Neuro/Ortho Spine Fellowship available for 7/1/2014